Angiosarcoma of the trunk of unusual presentation in an immuno-competent man.

Sir,

A 49-year-old male with no medical history, presented with a painful violaceous solitary nodule of the right side of 5 months duration. On physical examination, a violaceous indurated nodule measuring about 3cm × 2.5cm, with squamous mamillated surface was seen on the right side [Figure 1]. Examination of the whole tegument, nails, and oral mucosa was normal. There was no lymphadenopathy and the general state was conserved. There was no evidence of exposure to radiation. Viral serology markers including HIV were negative. A work-up for locoregional or metastatic metastases was negative. A histopathological examination of a skin biopsy revealed an irregular dissecting proliferation of anastomosing vascular channels invading the whole dermis without reaching the hypodermis [Figure 2a].

Figure 1

A violaceous indurated nodule, with squamous mamillated surface of the right side

Figure 2

(a) Irregular dissecting proliferation of anastomosing vascular channels invading the whole dermis (H & E stain, ×100). (b) The vascular channels are lined by abnormal endothelial cells (H & E stain, ×400). (c) On immunohistochemistry, the tumor cells were positive for CD 34, CD 31

These vascular channels were lined by abnormal endothelial cells with a low mitotic rate [Figure 2b]. Immunohistochemically, the tumor cells were positive for CD 34, CD 31 [Figure 2c]. An enlarged resection was realized subsequently, and margins of the excised lesion were free from tumor. A final diagnosis of cutaneous angiosarcoma of the trunk of low mitotic rate was thus rendered. The patient is currently well 3 months after the diagnosis of angiosarcoma.

Cutaneous angiosarcoma (CA) is a rare and aggressive endothelial-derived sarcoma.[12] This neoplasm mainly arise in three distinct clinical settings: 1) sporadic (involving face, scalp, and neck regions of elderly patients); 2) following long-standing lymphedema in relationship with Stewart–Treves syndrome or other acquired or congenital lymphedema causes; and 3) postirradiation.[12] It mainly occurs in males (sex ratio of 3:1), at an average age ranging from 60 to 80 years. CA arising among younger patients is frequently associated with risk factors (i.e., pre-existing vascular malformations or lymphedematous extremities).[1-5] Our patient, a 49-year-old man, was immunocompetent and had no risk factors. The most common locations are head (scalp 50%) and neck. In a retrospective American study of 69 CA and not associated with lymphedema or prior radiation, lesions occurred on the head/neck in 71%, on extremities in 21%, and on the trunk in 7%.[4]

The most common clinical features of CA are multifocal erythematous-to-violaceous nodules or masses. More rarely, the tumor presents as a solitary bluish or violaceous nodule, as an irregular ecchymosis or as a localized inflammatory edema as well.[5] CA may easily mimic lymphomas, carcinomas, neuroendocrine carcinomas, or adnexal malignant tumor.

Typical histological features of CA include a dissecting pattern with irregular proliferation of anastomosing vascular channels lined by abnormal endothelial cells. Other described patterns are solid epithelioid/spindled pattern with the presence of islands or diffuse sheets of epithelioid or spindled cells without formation of sinusoids or vascular channels and the mixed pattern with presence of a mixture of both histologic types.[3-5] Immunohistochemical and ultrastructural analyses can help particularly in undifferentiated forms of the tumor.

A multicentric study has assessed all clinicopathologic factors of 47 cases of CA in order to determine prognostic factors predictive of outcome. The authors concluded that histological grade and mitotic activity are poor markers of clinical aggressiveness. However, tumor size, depth of tumor invasion, and completeness of surgical resection are more reliable prognostic indicators.[5]

CA has high potential of locoregional and distant metastases despite aggressive multimodal therapy, mainly because of multifocality and unapparent spread of the tumor.

Our observation presents several particularities as follows:

the tumor occurs in a 49-year-old immunocompetent man, with no risk factors mainly of lymphoedema;

clinically, it presented as a solitary violaceous nodule of the trunk suggestive of lymphoma; and

the low mitotic rate of the tumour that requires aggressive treatments as well as close follow up as in CA with high mitotic rate.